Isolated fibrinogen deficiency is a particularly rare disorder in patients with normal preoperative prothrombin time/activated partial thromboplastin time.
A 62-year old female patient was admitted to hospital for curative lung cancer surgery. She had been diagnosed with adenocarcinoma, clinical stage T1bN0M0. Her medical history was unremarkable. Right Upper lobectomy with mediastinal lymph node dissection (MLND)was performed using video-assisted thoracoscopic surgery (VATS)by a thoracic surgeon who had significant experience in minimal invasive surgery. A right upper lobectomy with systemic medias-tinal lymph node dissection was performed. During the surgery,no blood transfusion was required estimated blood loss, 200 ml. She was tolerable to the postoperative pain and ambulated well in the general ward.
However, the PT INR was above 10 and aPTT was 32.6. A repeat test was performed, and the PT INR was found to be normal at 1.11. There Was no clinical condition that was suspected to raise the PT INRabove 10, and it returned to normal during the repeat test. Moreover, there was no active bleeding. Hence, the prolonged PT INRwas considered a technical error.
On postoperative day (POD) 1, the aPTT was once again prolonged to>180 seconds and the PT INR was>10. No active bleeding occurred. However, 2 packs of fresh frozen plasma (FFP) and vitamin k 20 mg were administered as a prophylaxis for bleeding.
On POD 2, PT and aPTT returned to normal again. The department of laboratory medicine was consulted to identify the cause of the changes in PT/aPTT seen over the last 3days. It was inferred that the result was most likely to be an analytical error. On POD 5, spontaneous bleeding was observed at the site of the chest drainage tube, and subcutaneous bleeding occurred on the back. The volume of fluid that drained from the chest tube increased.The patient's hemoglobin level decreased from 12.7 g/dL to 9.3 g/dL. On the evaluation of coagulation factors, it was found that the fibrinogen level was 39.8 mg/dL and isolated fibrinogen deficiency was diagnosed.Thereafter, the PT/aPTT was measured and fibrinogen tests were performed daily and the fibrinogen level was maintained at approximately 60 mg/dl.
Conclusively, the patient was diagnosed with isolated fibrinogen deficiency. Findings show that it is necessary to have a countermeasure for isolated fibrinogen deficiency in order to prevent any critical complications. In perioperative period, if repeated screening test for hemostasis is abnormal such as PT/aPTT prolongation, the mixing test with normal plasma is needed. After the mixing test, we should evaluate the clotting factor deficiency or the presence of inhibitors, and then confirm them by performing the clotting assay or further test to identify the type of inhibitor.
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